705 Case Report Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge Zeeshan Ahmad1, Qasim Stanazai2, Staphanie Wright3, Matthew Smolkin3, Patrick C. Ma4 1Section of Hematology/Oncology, Department of Medicine, West Virginia University School of Medicine, Morgantown, WV, USA; 2Department of Medicine, West Virginia University School of Medicine, Morgantown, WV, USA; 3Section of Molecular Pathology, Department of Pathology, West Virginia University School of Medicine, Morgantown, WV, USA; 4Penn State Cancer Institute, Penn State Health Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, PA, USA Correspondence to: Patrick C. Ma, MD, MSc. Professor of Medicine, Associate Director of Translational Research, Thoracic Oncology Disease Team Leader, Penn State Cancer Institute, Penn State Health Milton S. Hershey Medical Center, Pennsylvania State University, 500 University Drive, Hershey, PA 17033, USA. Email:
[email protected]. Abstract: Epithelioid sarcoma (ES) is an uncommon soft tissue neoplasm first described in 1970. It is a unique soft tissue neoplasm of adolescents and younger adults which usually presents as a subcutaneous and deep dermal mass in the distal portions of the extremities. The proximal-type variant of this rare soft tissue neoplasm was only recently reported. The proximal form typically arise in proximal extremities and in the deep parts of pelvis, perineum and genital tract. The proximal type variant has distinct histological characteristics and aggressive clinical course as compared to the distal ES. Inactivation of INI1 has been reported in both distal and proximal variants and can help to make the diagnosis. Furthermore, the proximal variant has a possible association with malignant rhabdoid neoplasm.